H. H. Malluche, Lexington, MD
Editor-in-Chief, Clinical Nephrology
Dear Dr. Malluche
We would like to submit the manuscript entitled "Clinicopathologic Features and
Treatment Response in Nephrotic IgA nephropathy with Minimal Change Disease”
which we wish to be considered for publication in Clinical Nephrology.
This is a large cohort study comparing the clinicopathologic patterns and outcomes
of the IgAN (IgA nephropathy) patients with nephrotic syndrome (NS) with or
without minimal-change disease (MCD). In this paper, 35.5% of 62 NS-manifesting
IgAN patients were reported as MCD-like pathological changes, which were
characterized
as
more
prominent
NS
manifestations
(heavier
proteinuria,
hypoalbuminemia and hypercholesterolemia), but slighter pathological changes.
These IgAN patients responded well to corticosteroid monotherapy but had a higher
rate of relapse. However, combination therapy of steroid and immunosuppressant
yielded a pretty high re-remission rate. Though manifesting with severe symptoms of
proteinuria, hypoalbuminemia, and hypercholesterolemia, they tended to have a
favorable clinical outcome probably due to the minimal pathological changes.
All authors have read and approved the final submitted version. The results
presented in this paper have not been published previously in whole or part,
except in abstract form. None of the authors have any financial or scientific
conflicts of interest with regard to the research described in this manuscript.
Thank you very much for your kindly consideration. Looking forward to hearing
from you.
Sincerely yours,
Xueqing Yu
Department of Nephrology
The First Affiliated Hospital, Sun Yat-sen University
Guangzhou 510080, China
Phone: 00862087335926
Fax: 00862087769673
E-mail: [email protected]
Clinicopathologic Features and Treatment Response in Nephrotic IgA
nephropathy with Minimal Change Disease
Jing Qin, MD1,2#, Qiongqiong Yang, MD, PhD
1,2#
, Xueqing Tang, MD, PhD
1,2
,
Wenfang Chen, PhD 3, Zhibin Li, PhD 4, Haiping Mao, MD, PhD 1,2, Zongpei Jiang,
MD, PhD 1,2, Fengxian Huang, MD, PhD 1,2, Xueqing Yu, MD, PhD1,2 *
1
Department of Nephrology, The First Affiliated Hospital, Sun Yat-sen University,
Guangzhou 510080, China,
2
Key Laboratory of Nephrology, Ministry of Health, Guangzhou, Guangdong, China,
3
Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University,
Guangzhou 510080, China,
4
Epidemiology and Clinical Research Unit, The First Affiliated Hospital, Sun Yat-sen
University, Guangzhou 510080, China
# Both of the authors contributed equally to this work
Running Title: Nephrotic IgA nephropathy with Minimal Change Disease
* Correspondence and offprint requests to Dr Xueqing Yu
Phone: 0086208766335
Fax: 00862087769673
E-mail: [email protected]
Word counts: abstract 250 words and manuscript 2565 words.
【Abstract】Objective: To analyze the clinicopathological features and therapeutic
response of nephrotic IgA nephropathy (IgAN) patients with minimal-change disease
(MCD). Methods: Sixty-two nephrotic IgAN patients were enrolled between January
2002 and December 2008, and divided into two groups including group A: patients
with MCD-like pathological features, and group B with non-MCD pathologic pattern.
The clinicopathological features, treatments responses were then analyzed. Results:
22 (35.5%) patients exhibited MCD-like pathological changes. Patients in group A
presented more prominent proteinuria, hypoalbuminemia and hypercholesterolemia,
but higher hemoglobin and a lower incidence of renal insufficiency compared to
group B (p<0.05). 41 patients were treated with corticosteroid, and the response rate
in group A is much higher than that in group B (90.0% vs. 67.5%, p=0.04), but the
relapse rate is much higher in group A (45.5% vs. 17.5%, p=0.02). 21 patients were
treated combining with immunosuppressant due to unresponsiveness or relapse, which
yielded a high re-remission rate in group A (87.5%). After follow-up of 53.9±26.9
months, the 5-year renal survival rate is higher in group A (100%) than that in group
B (80.9%), but no significant was observed (p=0.08). Conclusions: MCD-like
pathological changes exist in quite a few nephrotic IgAN patients. These IgAN
patients responded well to corticosteroid monotherapy but had a higher rate of relapse.
Though manifesting with severe nephrotic symptoms, they tended to have a favorable
clinical outcome probably due to the minimal pathological changes. Nevertheless,
larger sample size and longer follow-up period are needed for better understanding of
the disease.
【 Key words 】 IgA nephropathy-Nephrotic Syndrome-Minimal change disease
(MCD)-Treatment response
IgA nephropathy (IgAN) is the most frequent primary glomerulonephritis
worldwide, especially in China. It accounts for approximately 40%~50% of primary
glomerulonephritis[1, 2]. IgAN has various clinical and pathological manifestations
and corresponding considerable variations in prognosis. Most of IgAN patients
experience a mild to moderate proteinuria, but 5%~10% of them manifest as
nephrotic syndrome (NS)[2-5]. Among these minorities, there is a special subclass, of
which the pathological changes are similar to minimal-change disease (MCD).
Minimal-change disease is characterized with mild glomerular lesions under light
microscopy and extensive foot process fusion under electron microscopy. Few studies
have explored the differences in the clinicopathological features and prognosis of
these MCD-like IgAN patients compared with those with non-MCD-like pathological
changes.
In
this
study,
we
retrospectively analyzed
and
compared
the
clinicopathological features, treatment response, and prognosis of patients in these
two types of IgAN with NS.
Patients and Methods
Patients
From January 2002 and December 2008, a total of 1,215 Chinese patients were
diagnosed with primary IgAN at the First Affiliated Hospital of Sun Yat-sen
University, which accounted for 23.8% of all renal biopsies. The diagnosis of IgAN
was based on immunofluorescence microscopy showing mesangial IgA deposition as
the predominant or co-dominant immunoglobulin. Patients with Henoch-Scholein
purpura, systemic lupus erythematosus and liver cirrhosis were excluded. Among the
1,215 patients with IgAN, 104 patients (8.6%) presented with nephrotic syndrome,
which was defined by generalized edema, heavy proteinuria (more than3.5g/d),
hypoalbuminemia (less than 30g/L) and/or hypercholesterolemia (more than 200
mg/dL). With regular follow-up visits, 62 of 104 patients were finally enrolled into
this study and divided into two groups. Group A comprised 22 patients who exhibited
MCD-like pathological changes based on the following criteria: mild glomerular
lesions with mild or less proliferation of mesangial cells or matrix by light microscopy,
diffuse glomerular IgA-predominant mesangial deposition by immunofluorescence,
mesangial electron dense deposition and diffuse effacement of podocyte foot
processes by electron microscopy. The remaining 40 patients were enrolled in group B,
which did not exhibit MCD-like lesions.
Data Collection
The demographic and clinical data were reviewed retrospectively including age,
gender, medical history, blood pressure, body weight, body mass index (BMI),
serum triglycerides, cholesterol, plasma albumin, serum creatinine and blood urea
nitrogen, serum uric acid, serum IgA, urine protein and red blood cells, 24-h urinary
protein content, estimated glomerular filtration rate (eGFR, by the MDRD formula),
medication regimens, treatment efficacy, and prognosis.
Renal biopsy specimens were examined by light, immunofluorescence and
electron microscopy for the number of glomeruli and the presence of mesangial
hypercellularity, glomerulosclerosis, crescents, endocapillary proliferation, tubular
atrophy, interstitial fibrosis, foot process effacement (FPE) and the presence and
location of electron-dense deposits. Each specimen was also graded according to
Lee’s[6] and the OXFORD-MEST classification of gAN[7]. These were done
independently by two pathologists.
Treatment protocols
Corticosteroids were immediately administered to all the 62 patients when
diagnosed as NS, which referred to the administration of prednisone (or prednisolone)
at 1 mg/kg/day for eight weeks, and then tapered gradually. Combination therapy
(additional immunosuppressant including cyclophosphamide, mycophenolate mofetil,
or cyclosporine) were administered when considering unresponsiveness to 2-3 months
of prednisone (or prednisolone) treatment or relapse after tapering the corticosteroids.
Definitions
Complete remission (CR): urinary protein excretion less than 0.3g/24 h, a dipstick
test indicating trace or negative urinary albumin, normal serum albumin and stable
kidney function confirmed by serum creatinine within the normal range or not
increased by 15% more than baseline values. Partial remission (PR): a decrease of
urinary protein excretion in the range of 0.3~3.5 g/24 h or greater than 50% of
baseline level with serum albumin levels higher than 30 g/L and stable kidney
function. Non-remission (NR): urinary protein excretion that remained more than
3.5g/24 h or 0.3~3.5g/24h with serum albumin lower than 30 g/L and serum
creatinine levels increased by more than 30% of the baseline value. Relapse was
defined as the reappearance of significant proteinuria, defined as >1.0g/day
and/or >3+ urinary albumin by the dipstick test[3]. Renal end-point was defined as
End Stage Renal Disease (ESRD) in which dialysis or kidney transplantation was
needed.
Hypertension referred to systolic blood pressure ≥140 mmHg and/or diastolic blood
pressure ≥90mmHg (blood pressure readings were the mean of three independent
measurements that were obtained over at least 2 days). Chronic renal failure (CRF)
referred to conditions of kidney injury for longer than three months and serum
creatinine >133mol/L.
Statistical Analysis
Data were expressed as the mean ± standard deviation (normal distribution) or
medians with the 25th and 75th percentiles (non-normal distribution). Variables were
compared between the two groups using t-test and Mann–Whitney U-test. Differences
in the qualitative results expressed as frequency with percentage were compared using
chi-square test, Fisher’s exact test or the Kruskal-Wallis test as appropriate.
Kaplan-Meier survival curves were used to analyze cumulative renal survival.
Significance was defined as a P-value <0.05. Statistical analysis was performed using
SPSS version 16.0 (SPSS Inc., Chicago, IL, USA).
Results
Clinical Characteristics of Patients with Nephrotic Syndrome
Among the 62 IgAN patients with NS, 22 patients (35.5%, 28.6±16.9 years old)
were in group A, the other 40 patients (64.5%, 29.3±13.3 years old) were in group B.
The clinical characteristics of IgA nephropathy patients with NS at the time of renal
biopsy are shown in Table 1. No significant differences were detected for gender or
age distributions between the two groups (Table 1).
All these patients underwent renal biopsy within a median of 1.5 months of
symptom onset (0.5 ~ 5.3 months). The patients in group A exhibited much worse
clinical symptoms than those in group B, including more severe proteinuria (4.6±1.5
vs. 3.5±1.7 g/24 h, p=0.03), more apparent hypoalbuminemia (22.0±5.4 vs. 25.1±5.9
g/L, p=0.049), more pronounced hypercholesterolemia (9.6±3.1 vs. 7.9±2.6 mmol/L,
p=0.02), but higher hemoglobin levels (147.7±27.9 vs. 121.5±24.9 g/L, p<0.001). At
the time of renal biopsy, only two cases (9.1%) in group A were renal insufficiency
while there were 16 cases in group B (40.0%, p=0.02). Meanwhile, the incidence of
hypertension was lower in group A (4 cases, 18.2%) than in group B (14 cases, 35.0%)
although this difference was not statistically significant. Except for these, neither the
laboratory index including incidence of gross hematuria, levels of red blood cells in
the urine, nor the remaining clinical indices, including BMI, the course of the disease,
blood pressure, serum creatinine levels, serum uric acid, eGFR and serum IgA,
differed significantly between the two groups (Table 1).
Pathological Features of Patients with Nephrotic Syndrome
As shown in Table 2, group B had a significantly higher incidence of
glomerulosclerosis than group A. According to the OXFORD-MEST classification[7],
group A exhibited milder indices of mesangial proliferation (M), endothelial cell
proliferation (E), segmental sclerosis (S), and tubular atrophy/interstitial fibrosis (T),
with scores that were primarily M0 (59.1%), E0 (90.9%), S0 (63.6%), and T0 (95.5%).
In contrast, group B had scores that were primarily M1 (85.0%), E0/1 (55.0%/45.0%),
S0/1 (52.5%/47.5%) and T0 (72.5%). There were significant differences in the M, E,
T scores and interstitial lymphocytes and monocytes infiltration (p<0.05) between the
two groups. However, no significant differences in arteriole changes (arteriolar wall
thicken or hyaline degeneration) existed. According to Lee’s classification system[6],
group A primarily comprised patients in grade I–II (14 cases, 63.6%) while group B in
grade
III–V
(25
cases,
62.5%)
(p=0.001).
In
addition,
the
immunofluorescence intensity of mesangial IgA deposition in group A was
predominantly + to ++ (20 cases, 90.9%), whereas the intensity in group B was
predominantly ++ to +++ (30 cases, 75.0%), indicating that the mesangial IgA
deposition in patients of group A was considerably less severe (p=0.02).
Efficacy of Corticosteroids Monotherapy and Combinational Therapy
As shown in Table 3, all the 62 patients received corticosteroids at the start of
therapy and had a combined remission rate of 75.8%, which comprised a rate of
90.9% in group A and 67.5% in group B (p=0.04). However, group A had a much
higher relapse rate after corticosteroids treatment than group B (45.5% vs. 17.5%,
respectively, p=0.02). Due to the non-response or relapse of corticosteroid therapy, 8
patients in group A (8/22, 36.4%) and 13 in group B (13/40, 32.5%) which made to a
total of 21 patients finally underwent additional immunosuppressive treatment. And
no significant difference in the choice of the therapy scheme (corticosteroid or
combination therapy) between the two groups existed. For these 21 patients, the
combination therapy yielded an additional combined remission rate of 66. 7% (14/21),
in which group A was 87.5% while group B was 53.8% (p=0.17).
Long-term Clinical Outcome
The follow-up period for these 62 IgAN patients with NS was 53.9±26.9 months
(range 15.0~138.0 months). During this period, four deaths and five ESRDs were
reported in group B, resulting in a 5-year renal survival rate of 80.9%, whereas, none
ESRD or death was found in Group A, ending with a 5-year renal survival rate of
100% (p=0.08) (Figure 1). Interestingly, the combination therapy did not yield a
significant difference in renal survival rate when compared with steroids therapy in
either group A (p=1.00) or group B (p=0.76) (Figure 2). This result might indicate
that the pathological feature, instead of the therapeutic scheme, was associated with
the prognosis of IgAN patients with NS.
Discussion
Previous studies have reported that IgAN patients with NS typically present severe
pathological changes and indicate poor clinical outcome[8]. However, growing
clinical evidences have revealed that some of IgAN patients with NS respond well to
corticosteroid and have a favorable prognosis. Renal biopsy showed that in addition to
a typical feature of IgAN that diffuse IgA-dominant depositions in the glomerular
mesangial region, these patients have minimal changes in glomeruli by light
microscopy, and have diffuse effacement of podocyte foot processes and
mesangial electron dense deposition by electron microscopy. In other words, these
patients display MCD-like pathological changes. This special cohort of IgAN patients
was reported firstly in the 1980s by Mustonen et al., who discovered that three
NS-manifesting IgAN cases whose pathological alterations were reminiscent of MCD
along with a mesangial deposition of IgA[9]. After that, similar cases were gradually
reported, particularly among Asians. These cases have triggered a discussion
regarding the precise nature of this type of disease. Due to the intrinsic
clinicopathological diversity of IgAN, the initial predominant view considered the
disease to be IgAN[9-11]. Nonetheless, subsequent studies have revealed that patients
of this special cohort have a clinical behavior that is similar to MCD, and this has
prompted some researchers to argue that the disease belongs to an unusual type of
MCD, and mesangial IgA deposition is perhaps only an accompanying phenomenon
that does not play any direct role in the disease[12-14]. The others suggest that it
might a coincidental occurrence of MCD and IgAN, in which IgAN and MCD might
occur sequentially in a random order, and such a development may be only a
particular state at which the disease progresses to a certain stage[15]. Unfortunately,
previous studies were reports of individual patients, and the sample size was too small,
to serve as reliable clinical evidence. In our study, we systematically analyzed and
compared the clinicopathological characteristics, treatment protocols and clinical
outcome of two groups of IgAN patients manifesting as NS with or without MCD-like
changes and thus enable us to better understand these special cohort of IgAN.
Among the patients enrolled in this study, 35.5% of them displayed MCD-like
pathological changes. It is well known that hematuria, particularly recurrent gross
hematuria, is a common clinical manifestation of IgAN. However, the classic MCD
patients had an extremely low incidence of hematuria. Hence, the present study
measured hematuria in the two groups and found that group A had an incidence of
gross hematuria of 18.2%, and no statistically difference was existed between group A
and B. Previous studies have suggested that the IgAN-associated occurrence of
hematuria is related to mesangial cell and matrix proliferation that stems from
mesangial IgA deposition[16]. This might explain the finding that patients who had
mesangial IgA deposits in group A had a higher incidence of hematuria than the
classic MCD patients who did not have immune-complex deposits. And this further
suggested that mesangial IgA deposits may play a crucial role in pathogenesis.
By analyzing a variety of clinical indices, including proteinuria, hypertension and
renal function, our study revealed that the patients in group A had more severe
symptoms of proteinuria, hypoalbuminemia and hypercholesterolemia, but a lower
incidence of renal insufficiency and higher levels of hemoglobin. Previous studies
have indicated that elevated serum creatinine and hemoglobin are important clinical
indicators for monitoring the progression in IgAN patients[17, 18]. Specifically,
patients who suffer severe pathological renal damage have higher levels of serum
creatinine and lower levels of hemoglobin. The patients in group A had a lower ratio
of renal insufficiency, higher levels of hemoglobin and milder pathological changes.
This may be probably because that the mesangial IgA deposition in group A had not
yet reached the level that was sufficient to cause pathological changes like chronic
progressive IgAN (such as mesangial proliferation and sclerosis), which might be
associated with the merely slight mesangial IgA deposits observed in this group. As
such, the patients in group A behaved similarly to MCD-manifesting NS patients with
respect to their responsiveness to corticosteroid treatment and their prognosis (i.e.,
good corticosteroids response, high relapse rate, and favorable clinical prognosis).
Although this study was relatively larger than others that have focused on the IgAN
patients with NS, it still had several limitations. Firstly, it was a retrospective design
and the sample size was not large enough, which made the distribution of patient
numbers among the two groups non-uniform and could cause some bias in the results.
Whether our results can be generalized still needs to be further investigated. Secondly,
the follow-up period was not long enough, and some differences could not emerge.
For instance, a significant difference in the renal survival between the two groups
could not detected though it seemed to be lower in group B. Thirdly, no patient had a
renal rebiopsy after remission of NS, thus making the pathological changes unclear in
the patients after treatment.
In summary, a considerable number of the IgAN patients in our study who
exhibited clinical NS manifestations had MCD-like pathological changes. These
patients were characterized by severe clinical symptoms (e.g., massive proteinuria,
hypoalbuminemia and hypercholesterolemia). Although they responded well to
corticosteroids therapy, these patients had a relatively high relapse rate. However, the
combined corticosteroids and immunosuppressant treatment after relapse or
inefficiency to corticosteroids can yield a high additional remission rate and a
satisfactory clinical prognosis. Thus, MCD-like IgAN is indeed highly similar to the
typical MCD disease, except that the former has a higher likelihood of hematuria
which is probably related to mesangial IgA deposition. The deposition of IgA
complex on the mesangium may lead to proliferation of mesangial cells and matrix
and correspondingly aggravates the occurrence of hematuria. This atypical IgAN
resembles a special state in which disease might develop in different directions in the
presence of various stimuli. On one hand, the disease might progress into typical
IgAN under the continuous stimulation of IgA deposits coming from autoimmune
responses, on the other hand, the disease might be well controlled and stop
progressing any further. Nonetheless, the validity of this speculation depends on renal
rebiopsy, which will enable us to elucidate the disease progression. After all, larger
sample size, longer follow-up period as well as more clinical indices, especially the
rebiopsy, is needed for better understanding of the disease.
Table 1 Comparison of the clinical characteristics of nephrotic IgA nephropathy with
or without MCD-like features
All patients
Group A
Group B
(n=62)
(n=22)
(n=40)
29.1±14.5
28.6±16.9
29.3±13.3
<20
16 (25.8%)
6 (27.3%)
10 (25.0%)
20-30
24 (38.7%)
10 (45.5%)
14 (35.0%)
31-40
9 (14.5%)
2 (9.1%)
7 (17.5%)
41-50
6 (9.7%)
1 (4.5%)
5 (12.5%)
> 50
7 (11.3%)
3 (13.6%)
4 (10.0%)
34 (54.8%)
15 (68.2%)
19 (47.5%)
0.18
1.5 (0.5 , 5.3)
1.35 (0.3 , 6.0)
1.5 (1.0 , 4.8)
0.35
Follow-up period (mons)
53.9±26.9
53.4±20.3
54.3±30.2
0.67
Gross haematuria (n, %)
9 (14.5%)
4 (18.2%)
5 (12.5%)
0.71
Hypertension (n, %)
18 (29.0%)
4 (18.2%)
14 (35.0%)
0.15
Systolic BP (mmHg)
124.5±18.1
120.7±14.9
126.6±19.6
0.23
Diastolic BP (mmHg)
78.4±10.9
75.6±8.9
80.0±11.6
0.13
MAP (mmHg)
93.8±12.5
90.6±9.8
95.5±13.5
0.60
BMI(kg/m2)
21.6±3.8
21.9±4.1
21.4±3.7
0.14
Urinary protein (g/24 h)
3.9±1.7
4.6±1.5
3.5±1.7
0.03
Serum Albumin (g/L)
24.0±5.9
22.0±5.4
25.1±5.9
0.049
Cholesterol (mmol/L)
8.5±2.9
9.6±3.1
7.9±2.6
0.02
Triglycerides (mmol/L)
2.0±0.9
1.9±1.0
2.0±0.9
0.71
93.0 (68.8,152.3)
81.5 (67.8,98.3)
102.5 (68.3,165.7)
0.13
18 (29.0%)
2 (9.1%)
16 (40.0%)
0.02
92.2±59.6
110.0±58.9
82.5±58.4
0.07
Uric acid (umol/L)
417.1±148.0
442.5±171.1
403.7±134.8
0.35
Hemoglobin (g/L)
130.8±28.7
147.7±27.9
121.5±24.9
<0.001
2.4±1.0
2.2±0.8
2.6±1.1
0.11
Age (years)
P-value b
0.54
Age
0.74
distribution
Men (n, %)
Interval between presentation
and biopsy (mons)
Serum creatinine (umol/L)
Serum creatinine
> 133umol/L (n, %)
eGFRa (mL/min/1.73m2)
Serum IgA (g/L)
RBC in urine (n, %)
42(67.7%) /
18(81.8%) /
24(60.0%) /
＜2＋ / ≥ 2＋
20(32.3%)
4(18.2%)
16(40.0%)
0.10
Note: BP, blood pressure; RBC, red blood cell; a eGFR (mL/min/1.73 m2) = 175 ×
(plasma creatinine)−1.234× age−0.179× 0.79 (if female)
[19]
.
b
p value: comparison
among group A (nephrotic IgA nephropathy with MCD-like features) and group B
(nephrotic IgA nephropathy without MCD-like features). p values of <0.05 were
considered significant.
Table 2 Comparison of the pathologic features of nephrotic IgA nephropathy with or
without MCD-like features
Group A (n=22）
(n ,%)
Group B (n=40)
(n ,%)
P-value a
5,22.7%
21,52.5%
0.02
Mesangial hypercellularity M0/M1
13,59.1%/9,40.9%
6,15.0%/34,85.0%
＜0.001
Endocapillary hypercellularity E0 /E1
20,90.9%/2,9.1%
22,55.0%/18,45.0%
0.004
Segmental glomerulosclerosis S0/S1
14,63.6%/8, 36.4%
21,52.5%/19,47.5%
0.40
21,95.5%/1,4.5%
29,72.5% /11,27.5%
0.04
16,72.7%
5,22.7%
1,4.5%
0
0
14,35%
17,42.5%%
1,2.5%
6,15.0%
2,5.0%
0.02
arteriolar wall thicken
6,27.3%
15,37.5%
0.42
arteriolar hyaline degeneration
5,22.7%
9,22.5%
1.00
8,36.4%/12,54.4%
6,15.0%/19,47.5%
2,9.1%/0
11,27.5%/4,10%
I
9, 40.9%
3,7.5%
II
5,22.7%
10,25.0%
III
8,36.4%
13,32.5%
IV
0
12,30.0%
V
0
2,5.0%
1. Glomerular lesions
Global glomerulosclerosis
2. Tubulointerstitial lesions
Tubular atrophy/interstitial fibrosis
T0/T1~2
Lymphocytes and monocytes infiltration
none
≤25%
25%～50%
50%～75%
＞75%
3. Arteriolar changes
4. IgA glomerulus
immunofluorescence
+ /++
0.02
+++ /++++
5. Lee’s classification
a
0.001
Note: p value: comparison among group A (nephrotic IgA nephropathy with
MCD-like features) and group B (nephrotic IgA nephropathy without MCD-like
features). The degree of M, S, E, T was defined by Oxford classification [4]. p values of
<0.05 were considered significant.
Table 3 The response to corticosteroids and/or immunosuppressant therapy in the
patients with nephrotic IgA nephropathy with or without MCD-like features
All patients
(n ,%)
Group A
(n ,%)
Group B
(n ,%)
n=62
n=22
n=40
Complete remission
28,45.2%
15,68.2%
13,32.5%
Partial remission
19,30.6%
5,22.7%
14,35.0%
Non remission
15,24.2%
2,9.1%
13,32.5%
Total remission
47,75.8%
20,90.9%
27,67.5%
0.04
Relapse
17,27.4%
10,45.5%
7,17.5%
0.02
n=21
n=8
n=13
Treatment
1.Corticosteroids
therapy
p-value a
0.79
0.02
0.79
2. Combination therapy
(21/62,33.9%) (8/22,36.4%) (13/40,32.5%)
Complete remission
10,47.6%
6,75.0%
4,30.8%
Partial remission
4,19.0%
1,12.5%
3,23.1%
Non remission
7,33.3%
1,12.5%
6,46.2%
Total remission
14,66.7%
7,87.5%
7,53.8%
0.16
0.17
Note: a p value: comparison among group A (nephrotic IgA nephropathy with
MCD-like features) and group B (nephrotic IgA nephropathy without MCD-like
features). p values of <0.05 were considered significant.
Fig. 1 Kaplan–Meier survival curves for the nephrotic IgA nephropathy patients with
or without MCD-like features. Log rank significance for ESRD =0.08.
Fig. 2 Kaplan–Meier survivals curve for therapeutic outcome in the nephrotic IgA
nephropathy patients with or without MCD-like features. Log rank significance for
ESRD in Group A= 1.00, Log rank significance for ESRD in Group B =0.76, Log
rank significance for ESRD in Total =0.89. ISD: Immunosuppressant; Pred:
Prednisone.
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